take place in the sinuses in a way different from the ordinary
post-mortem clots, but this was looked upon rather as an anatomical
curiosity than as a fact of practical importance and clinical
significance, and it is chiefly among observers of the present century
that we find a growing knowledge of the conditions under which it
occurs and the symptoms to which it gives rise.
Thrombi in the cerebral sinuses are not essentially different from
those formed elsewhere, and the reader is referred to the account
given in the article on General Pathology for a history of their
formation, growth, appearances, and transformations. For our purposes
it is sufficient to recall that they may be white, red, or striated,
either partly or wholly obstructing, and that they may become
degenerated and partly or wholly washed away. The most important
distinction of all, however, is that into two classes, of which the
first consists of those which are simply depositions of fibrin in a
comparatively healthy vessel, and the second of those which are
dependent on a phlebitis.
In order that a thrombus may form it is necessary that there should
be, in the first place, a special condition of the walls of the
veins—not necessarily, however, inflammation, though this is one of
the most frequent and probably the most active form; second, a
slackening of the blood-current; and, third, perhaps a peculiar state
of the blood, though this latter is not certain. A thrombus tends
strongly to grow, and when already formed furnishes a most favorable
point for the deposition of more fibrin.
The cerebral veins furnish a very suitable place for the coagulation
of the blood for several reasons: they are roomy in proportion to the
amount of blood they carry; they are tortuous and abundantly
anastomosing, so that the current of blood is almost reversed at some
points, and can easily stagnate; the veins of the diploë are held open
by their bony walls, and the sinuses by their stiff membranous ones,
so that they cannot collapse and thus limit the extension of a
thrombus once formed.
The sinuses most frequently affected, though none are free from the
liability, are the cavernous, superior longitudinal, and lateral.
The results of thrombosis of the sinuses and veins are not equivalent
to those of a similar process in the arteries, and they may be said in
a general way to be more diffused, as might be expected from the much
greater freedom of anastomosis. Limited softening is rarely a
consequence of occlusion even of a considerable number of veins, but
it has been observed. A large area of softening of one hemisphere, not
involving the temporal and occipital lobes, has been seen with
thrombosis of the parietal veins[58] (the internal capsule and ganglia
were not affected).
[Footnote 58: _Gaz. des Hôp._, 1880, 1066.]
Passive congestion in the brain, as elsewhere, although apparently
entirely incompatible with the normal function, seems to be able to
sustain a low form of structural integrity.
Bleeding may take place from the congested veins behind the
obstruction, constituting a distinct form of cerebral hemorrhage which
does not depend upon an arteritis, although if miliary aneurisms were
present the {983} occurrence of thrombosis would undoubtedly tend to
their rupture. The writer, however, is not aware of such a coincidence
having been actually observed. Hemorrhages are usually diffuse,
composed of or accompanied by a number of small effusions, and
situated on or near the surface of the brain or distinctly meningeal.
Punctiform hemorrhages are exceedingly common.
Phlebitis of the cerebral veins is very likely to run into meningitis,
and the two affections are often so closely united that it is
difficult to say which was the first. Œdema is a consequence of venous
obstruction in the brain as well as elsewhere, and is seen also around
some of the peripheral veins connected with the sinuses.
ETIOLOGY.—Venous thrombosis in the brain depends chiefly on three sets
of causes, though it must be admitted that there are a few cases where
the origin cannot be distinctly traced and where no previous disease
has existed. In the marantic form, occurring chiefly in the very old
and in children, as well as in cases of wasting and depressing
diseases in adults, a simple thrombosis without inflammation takes
place. Two conditions, and sometimes three, are combined here to
produce the result—feebleness of the blood-current from a
corresponding state of the heart, diseased endothelium of the vessels
from defective nutrition, and possibly, where profuse watery
discharges have been going on for some time, an increased tendency to
coagulation from the inspissation of the blood.
Rilliet and Barthez and Von Dusch[59] give the following tables of
ages at which this form of thrombosis has been observed. The
observations of the former were made in a children's hospital, and
hence do not affect the question of its frequency in later life.
Perhaps the rules of admission may account for the absence of cases
under one year of age, of which Von Dusch collected several:
Rilliet and Barthez. Von Dusch.
Under 1 year . . . . . . . . . ... 5
2 years . . . . . . . . . . . 2 1
4 " . . . . . . . . . . . 4 1
5 " . . . . . . . . . . . 1 1
6 " . . . . . . . . . . . 1
7 " . . . . . . . . . . . 1
9 " . . . . . . . . . . . 2
10 " . . . . . . . . . . . 1
11 " . . . . . . . . . . . 1
12 " . . . . . . . . . . . ... 1
14 " . . . . . . . . . . . ... 1
Adults (20, 23, unknown) . . . ... 3
53 years . . . . . . . . . . . ... 1
Aged women . . . . . . . . . . ... 2
[Footnote 59: Sydenham Society's translation.]
The special diseases in which thrombosis is most likely to be met with
are given by Bouchut as follows. The same remark is to be made about
these as about those of Rilliet and Barthez. The table is given as
convulsions from thrombosis of sinuses:[60]
Chronic enteritis . . . . . . . . . 5
Measles and catarrhal pneumonia . . 2
Chronic pneumonia . . . . . . . . . 5
Phthisis . . . . . . . . . . . . . 8
Anasarca without albuminuria . . . 1
Chronic albuminuria . . . . . . . 2
Whooping cough and pneumonia . . . 7
Scrofula, tubercle of bones, etc. . 1
Gangrene of mouth . . . . . . . . . 1
Diphtheritis . . . . . . . . . . . 2
Von Dusch gives a number of cases of the same kind, as do many
subsequent writers, but without tabulation. Virchow[61] reports a case
of congenital variola with thrombosis of the sinuses of the dura
mater, the superior and inferior cava, and vessels of the cord.
[Footnote 60: _Gazette des Hôpitaux_, 1879.]
[Footnote 61: _Arch._, 1859, 367.]
{984} It is probable that simple anæmia may, here as elsewhere, either
alone or with other debilitating influences, lead to thrombosis. Von
Dusch remarks that quickly operating and debilitating influences lead
to thrombosis, and gives as an instance a case where a puerperal
peritonitis, for the cure (?) of which repeated copious abstractions
of blood were made during nine days, was supposed to be the cause. The
puerperal condition seems to have a tendency in this direction in a
way not always to be explained by the ordinary rules of the
transmission of emboli or of phlebitis. Although in those reported by
Ducrest[62] phlebitis of the pelvic veins existed or was suspected, in
the first of these five cases the lesion may have been, so far as the
description goes, arterial instead of venous thrombosis; and in the
second it is possible that the succession of events was uterine
phlebitis (with the addition of a large sacral slough), lobular
pneumonia surrounded and traversed by veins which were affected with
phlebitis, emboli in the arteries of the cortex, and consequent venous
thrombosis. In the third, fourth, and fifth the connection between the
uterine phlebitis and the inflammation of the cerebral veins (in two
cases meningitis) cannot easily be made out, except by the rather
vague assumption of a general tendency to phlebitis, which was shown
in one by a similar condition in the vein of the arm where the patient
was bled. Empyema has been followed by hemiplegia, cerebral softening,
and thrombosis of the lateral sinus. The venous thrombosis in such a
case may be secondary.
[Footnote 62: _Archives générales_, 1847, p. 1.]
Marantic thromboses are more likely to occur upon one side, and that
the side upon which the patient habitually lies.
The second class of cases embraces those where a simple obstruction,
partial or complete, of the current of the blood gives the
starting-point for a thrombus in the veins. Such an obstruction may be
formed by an embolus, but in the veins this cannot be considered an
important factor, although a portion of a thrombus may be detached and
become lodged in a narrower vessel or branch farther along. In this
way the propagation of thrombosis for a short distance toward the
heart may be accounted for.
A tumor or inflammatory exudation may press upon a vein or intrude
into it, but most cases of obstruction-thrombosis are traumatic in
origin. Thromboses arising in connection with tubercular meningitis
may be looked upon as having both an obstructive and marantic cause.
In many wounds of the vertex, gunshot and other, the walls of the
superior longitudinal sinus are pressed upon by pieces of bone, and
sometimes spiculæ have directly penetrated it. This class of injuries
is also likely to cause phlebitis without any actual penetration or
compression of the sinus, simply as a result of the inflammation of
tissues in the neighborhood. The thrombi formed in these cases are not
necessarily completely occluding. Where direct injury to the sinus or
in its immediate neighborhood gives rise to phlebitis and consequent
thrombosis, we have a condition closely resembling that of the third
class, where disease of an inflammatory character in the tissues of
the skull, neck, or face sets up a phlebitis and thrombosis which are
transmitted to the intracranial veins and sinuses.
The most frequent source of this third form of inflammatory thrombosis
{985} is the chronic inflammation of the middle ear with the mastoid
cells. The inflammation may be propagated through a carious or
necrosed portion of the temporal bone to the petrosal and lateral
sinuses, or may, without disease of the bone, be carried by the small
veins which open into the sinuses from the petrous and mastoid portion
of the temporal in this region. Abscesses in the neck may set up a
phlebitis extending up the jugular to the lateral sinuses, to which a
meningitis may possibly be added.
Carbuncles about the root of the nose, face, and so far down as the
upper lip are very prone to give rise to thrombosis propagated through
the ophthalmic vein to the cavernous sinus; and it is probably this
risk which gives to carbuncles in this situation their well-known
peculiar gravity. The divide or watershed between the regions which
drain backward through the cranium and those which are connected with
the facial vein below is apparently situated about the level of the
mouth, so that a carbuncle of the lower lip is much less dangerous.
Billroth, however, gives a case where a carbuncle in this situation
was followed rapidly by cerebral symptoms and death, and where a
thrombo-phlebitis was not improbable. He mentions another case where a
carbuncle upon the side of the head set up an inflammation which
travelled along a vein into the cellular tissue of the orbit, and
thence through the optic foramen and superior orbital fissure into the
skull.
Erysipelas of the scalp apparently causes phlebitis in some cases, and
even eczema in the same situation seems to have done so. When the
erysipelas is situated about the upper part of the face, the path of
transmission is through the ophthalmic vein; but when upon the vertex,
it may be propagated through the small veins that penetrate the bone.
This result is certainly a rare one in facial erysipelas of the
ordinary and superficial kind, which is a notoriously benign disease
for one of such apparent severity. It may, however, be more frequent
than ordinarily supposed, since cerebral symptoms occasionally appear
at a date too late to be accounted for by the fever and too slight to
be referred to extensive interference with the cerebral circulation;
the lesion to account for which, as they do not cause death, can be
only inferred, though it is not unreasonable to suppose it to be a
limited thrombosis.
Dowse[63] describes the case of a robust man who fell on the back of
his head, but walked home. After a few days he had a severe headache,
chill, and total loss of vision. His temperature rose; he had
erysipelas and partial coma, but no convulsions. There was thickening
of the scalp, but no fracture of the skull and no adhesions of the
membranes. The superior longitudinal lateral sinuses were free from
thrombi, though there was a roughness about the latter, as if there
had been a fibrinous deposit. The cavernous sinuses were almost
completely occluded with adherent fibroid masses, and there was
hemorrhage in the anterior lobe. There was some degeneration of the
brain-structure, but no disease of the arteries.
[Footnote 63: _Trans. Clin. Soc._, 1876.]
Ulcerations in the nasal passages and ozæna have proved
starting-points for thrombosis.[64]
[Footnote 64: _Med. Times and Gaz._, 1878, i. 614.]
Thrombosis of the jugular veins and corresponding cavernous sinus,
with paralytic symptoms, has been observed in the horse.
{986} The symptoms produced by venous thrombosis, as might be supposed
from their varying location and extent, and also from the fact of
their being almost invariably connected with other diseases having
marked and severe symptoms of their own, are not always easy to pick
out from among many others, but they are sometimes very well marked
and characteristic. A distinction must obviously be made between the
symptoms of simple thrombosis depending on interruption of the
cerebral circulation and those of phlebitis, which give rise in
addition to febrile phenomena common to phlebitis in any of the large
veins.
The symptoms which indicate venous obstruction, without reference to
its inflammatory or non-inflammatory character, are of two kinds:
first, those dependent upon the disturbance of the functions of the
brain; and, secondly, those which depend upon congestion and
compression of other structures.
According to the locality and completeness of the obstruction we meet
with brain symptoms.
In the marantic thrombosis of children these may be very vague, and
consist either in restlessness, followed by somnolence and coma, or,
most especially, in convulsions. The convulsions may be partial and
involve the face only; they may affect one side only, or, what is more
usually the case, be general. There is almost always strabismus. There
may be conjugate deviation. This latter phenomenon is said by Bouchut
to be of no value in children, as it may take place in either
direction, from or toward the lesion, but possibly the distinction
between the spastic and paralytic forms was not duly observed by him.
The condition of the fontanelles is spoken of as yielding and
depressed, with the edges of the bones overlapping. They may, however,
become again tense in the course of the disease from exudation or
hemorrhage taking place. Paralysis is not so marked as in adults, but
may be present.
In adults delirium takes the place of convulsions, due to a
disturbance of circulation over a considerable area, rather than to a
total suppression in a more limited one. Paralyses are not
infrequently met with, either in the form of a hemiplegia or more
localized. Hemorrhage will naturally be followed by its usual
consequences, according to its location. Headache, often very severe,
is among the early symptoms.
It is evident that none of these symptoms can be considered highly
characteristic. They can only furnish a certain amount of probability
in cases where the general course of the disease has made it likely
that thrombosis may take place.
There is another set, however, which, when present, offer the
strongest kind of confirmation: these are due to the pressure from the
veins themselves.
Œdema about the points at which the intracranial circulation is
connected with that of the face and neck may give rise to protrusion
of the eyeball, conjunctival ecchymoses, swelling of the upper lip,
and even of the upper part of the face, which sometimes becomes
slightly cyanosed from the congestion. Epistaxis has been noted. Œdema
may be noticed about the mastoid process when the thrombosis is
situated in the lateral sinuses, but it would be important in many
cases to distinguish this from inflammatory œdema directly due to
disease of the bone.
Œdema of the optic disc, as shown by obscuration of its outlines, with
large and pale vessels, has been observed by Bouchut.
{987} Veins closely connected with those within the cranium may be
thrombosed, and felt as hard cords by the finger. This may occur in
the facial veins about the orbit, in those around the mastoid, or in
the jugulars. On the other hand, if one cavernous sinus is filled with
a coagulum which does not go down into the jugular, this vein will
naturally be empty or receive only a small amount of blood from other
veins.
When the cavernous sinuses are affected, we are likely to have a set
of phenomena due to the pressure of the clot upon the nerves which
pass through it—_i.e._ the third and fourth, part of the fifth and
sixth—with filaments of the sympathetic accompanying the carotid
artery. Hence dilatation of the pupil, strabismus, or ptosis, and
other ocular paralyses may be the symptoms observed.
It is possible that a headache upon the side of the affected sinus may
be due to vascular dilatation from paralysis of the sympathetic, or to
a direct pressure upon the first branch of the fifth pair.
DIAGNOSIS.—The diagnosis of venous thrombosis may be almost entirely a
conjectural one in those cases where the cerebral symptoms are vague
or mixed with others peculiar to the causative disease. Where wasting
disease has existed, the patient is much emaciated, and profuse
discharges have diminished the fluidity of the blood, the rapid
supervention of coma with slight spasms or general convulsions will
render it highly probably that thrombosis is taking place. Unilateral
symptoms would greatly increase this probability, and if any
accessible veins about the head, neck, or face could be definitely
distinguished as filled with firm coagula, the diagnosis would
approach certainty.
In cases of this kind the only condition likely to put on the
appearance of thrombosis is the simple inanition or so-called
hydro-encephaloid disease, which comes on in exactly the same sort of
cases. Localized phenomena must be the chief point of difference.
Fortunately, the distinction is practically not an important one.
In wounds of the vertex affecting the longitudinal sinus the question
likely to arise where cerebral symptoms supervene is that of
thrombosis or abscess. Here the more definite localization is likely
to be upon the side of the abscess, although, as is well known, this
may remain latent or nearly so for a considerable time, and in general
is much more chronic in its course than thrombosis.
The swelling of the external veins, epistaxis, œdema of the lid,
protrusion of the eyeball, with œdema of the optic papilla, with only
moderate fever, would favor the diagnosis of thrombosis, while optic
neuritis, if present, with chills, would render the abscess more
probable. Unless the wound were sufficiently severe to fracture a
piece of bone into the sinus, or unless the subsequent inflammation
were of an unhealthy character, the abscess in a person of middle age
and previous good health may be considered the more probable of the
two. In the case of Dowse, already mentioned, the diagnosis between
abscess and thrombus must have been very difficult, and, as it seems
to the writer, would have been more likely to rest upon abscess or
meningitis than upon the condition afterward found to exist.
Where inflammatory diseases exist which are known to lead to
thrombosis with phlebitis, the practitioner, if on the lookout, can
often make a diagnosis with a high degree of probability in its favor.
The cerebral {988} symptoms with the venous swelling, collateral
inflammatory œdema in the more immediate neighborhood of the lesion,
and slight œdema and congestion at more distant points, and a febrile
movement indicating a distinct inflammatory exacerbation, will point
very strongly to thrombo-phlebitis.
An absolute distinction between such a condition of the veins and a
meningitis arising under exactly the same circumstances may not always
be possible, and is the less important since the two affections are
likely to coexist and form a part of the same disease.
The localization of the thrombus is to be determined partly by the
paralytic symptoms, if such exist, but principally by the situation of
the secondary œdema and from the lesion which forms the
starting-point. It has been said that the jugular vein of the side on
which thrombosis exists is less full; and this point might be of value
when the lateral sinus is affected.
PROGNOSIS.—From the character of the lesion itself, as well as from
the diseases with which thrombosis is usually connected, it will
readily be seen that the prognosis is in general a highly unfavorable
one; but it is possibly regarded as too inevitably so, for the reason
that a positive diagnosis may be in slighter cases a matter of
considerable uncertainty, so that the practitioner, even if attempting
to make an accurate anatomical explanation of obscure cerebral
symptoms, is as likely to think that he has been mistaken as that his
patient has recovered from so serious a disease.
Cases, however, have been reported where the diagnosis seems as clear
as it can be made without an autopsy, and recovery has taken place.
A case is reported by Voorman[65] of a child aged six months who had
diarrhœa and vomiting, much prostration, sunken fontanelles,
overlapping cranial bones, trembling of the tongue, slight spasm of
the right arm and leg, head drawn back, and strabismus. The head
afterward increased in size, the temporal vein was swollen and hard,
with œdema of the skin in its neighborhood. There was gradual
improvement and recovery, though when the patient was four and a half
years old its mental development corresponded to that of a child two
years younger.
[Footnote 65: _Centralb. f. d. Med. Wis._, 1883.]
In another, by Kolb,[66] a child of seventeen, well nourished, had a
purulent discharge from the right ear. Besides headache, delirium,
hyperæsthesia, convulsions, and then sleepiness and loss of
consciousness, the following symptoms pointed toward thrombosis of the
sinuses: Chills, inflammatory swelling over the right mastoid, with
fulness of a cutaneous vein passing over it; a purely œdematous
swelling in the neighborhood of the internal jugular or temporal
fossa, forehead, and both upper eyelids, with exophthalmos on the
right side; photophobia, blepharospasm, and cloudy vision; nose-bleed.
There was no elevation of temperature, and recovery took place.
[Footnote 66: _Berl. klin. Woch._, Nov. 13, 1876.]
A case of thrombo-phlebitis following otitis and terminating in
recovery is reported at length by Wreden.[67]
[Footnote 67: _Archives of Ophth. and Otol._, 1874, lii.
(translation).]
The PROGNOSIS in any particular case can be based only on the severity
of the symptoms and on the character of the preceding disease.
TREATMENT.—The prophylaxis of this affection evidently consists in
{989} the proper treatment of the diseases upon which it depends, and
might therefore be made to embrace nearly the whole range of tonic,
roborant, antiphlogistic, and antiseptic measures, to say nothing of
surgery and obstetrics. The proper nourishment of infants and
children, the cutting short, when possible, of their acute diseases,
or preventing their debilitating effects, will reduce marantic
thrombosis to a minimum. At a later period of life the proper surgical
management of carbuncle, abscess in the neck, and of the puerperal
condition will tend to avoid this risk.